In the United States there are approximately 36,000 new cases of kidney cancer each year, and 12,000 individuals die from the disease. Kidney cancer is an International disease. New syndromes are being recognized and characterization of the associated symptoms as well as identification of the genes involved in the disease are important to develop appropriate therapies. Continuation of this project is of great importance because at the moment we have families with kidney cancer that do not have any of the genetic changes known, and therefore, we need to studied them morphologically and genetically, as well as to try to identify new proteins useful as tumor markers. Specific aims of our study are: 1- To define the types and morphological characteristics of new renal syndromes. 2) To identify genes and molecular changes responsible for these new syndromes 3) Identify genotype/ phenotype correlation 4) To study the associated lesions such as uterine and other smooth muscle tumors, skin, lung and ovarian tumors, and investigate if they share similar genetic changes. Continuation of this research is essential, first because these tumors can only be recognized by their morphology and second because we lack both, markers for early detection and good forms of therapy.